Swayam P Nirujogi, MD, MBA

You are asked to evaluate a healthy 7-year-old boy with an asymptomatic brown “dirt-like” rash on the anterior neck (Figure 1). It has progressed over the last 2 months and does not clear with routine bathing and aggressive scrubbing by his mother with soap and water. After wiping with 70% isopropyl alcohol in the office, the brown pigment readily disappeared and mild erythema secondary to rubbing was noted confirming the diagnosis of Terra firma-forme dermatosis (Figure 2).

Terra firma-forme dermatosis (TFFD) is an acquired, benign, idiopathic dermatosis first described by Duncan and his colleagues in 1987. The name is derived from the Latin phrase ‘terra firma’ which refers to ‘dry land’. It is a rare type of keratinization disorder characterized by an asymptomatic brownish gray or black dirt-like patch that cannot be removed with soap and water and cleansers, but needs wiping with isopropyl alcohol or ethyl alcohol. The highest prevalence was found among prepubertal children (88.6%) with an average age of onset of 10.4 ±7.5 years, ranging from 3 years to 72 years.

 Commonly, the skin on the trunk and neck was involved, about a quarter of all patients showed involvement of the extremities, and in less than 3% over the head. Most of the lesions are solitary: However, in about 30% several lesions were found. It is often underreported.

Exact etiopathogenesis is not well understood. It is believed that the lesions arise as a result of a delay in the maturation of keratinocytes, with melanin retention and a sustained accumulation of sebum, sweat, corneocytes, and microorganisms in regions in which the skin is more sensitive to trauma and hygiene measures are less vigorous (neck, navel, flanks) leading to insufficient exfoliation and the formation of a highly adhesive, compact dirt like scale.

 It explains the hyperkeratosis and hyperpigmentation clinically seen in TFFD.

Histopathological examination, rarely required, shows prominent lamellar hyperkeratosis with whorls, keratotic plugging of follicular orifices, keratin globules in the stratum corneum, papillomatosis, and sharp invaginations between the papillae, increased melanin pigment in the basal layer, and minimal lymphocytic liquefaction. A Fontana-Masson stain shows focally increased melanin pigment in the basal layer of the epidermis. Periodic acid Schiff stain revealed a few vellus hair shafts with pityrosporum spores.

Terra firma-forme dermatosis is characterized by accumulation of hyperpigmented, hyperkeratotic and verrucous scale and/or crusts. Rarely palpable verrucous or papillomatous plaques, reticulated patches, and mild focal scaling have been observed.

Dermoscopy shows large, polygonal, plate-like, brown scales in a mosaic, cobblestone, or tie like pattern interrupted in furrows. Diagnosis can be confirmed by rubbing the lesion with an alcohol-soaked pad or cotton ball, which results in resolution of the lesions.

 This is known as the skin modified by alcohol rubbing test.

Dermatosis neglecta typically affects individuals of any age with neglected hygiene.

 The brownish, hyperkeratotic, verrucous crusts and corn flake-like scales, often symmetrical, result from progressive accumulation of sebum, cellular debris, keratin, sweat, and exogenous impurities. Lesions can be removed with soap and water or an alcohol swab or cotton ball. Histopathologically shows lamellar hyperkeratosis without any whorled hyperkeratosis.

Confluent and reticulated papillomatosis (CARP): CARP is an acquired icthyosi-form dermatosis characterized by persistent, asymptomatic, dark, scaly macules and patches that tend to be confluent in the center and reticulated at the periphery and localized predominantly on the trunk.

 The lesions are red to brown, slightly verrucous papules on chest, back, neck, chin, arms, axillae. It may respond to topical therapy with topical calciportiol or retinoids but responds best with oral doxycycline.

Post-inflammatory hyperpigmentation also known as post inflammatory melanosis, develops as a sequela of a variety of insults to the skin, such as atopic dermatosis, acne vulgaris, trauma, chemical/physical injuries. It presents as hyperpigmented macules/patches in the preceding inflammatory dermatosis or injury. The color tends to be tan to light brown if the excess pigment is within the epidermis. If the excess pigment is in the dermis, the color is usually blue or dark gray.

 Acanthosis nigricans consists of dark, velvety thickening of the skin, usually on the nape and sides of the neck, as well as the Axillae and inner thighs. The lesions typically presents in a symmetric fashion. There is no melanin deposition. Rather, the pigmentation is mainly due to hyperkeratosis. The condition is most commonly caused by obesity, metabolic syndrome, diabetes mellitus. The hyperpigmentation or dust cannot be removed by washing with soap and water or alcohol swab or cotton ball.

The condition of TFFD can be treated by forceful rubbing with gauze immersed into 70% isopropyl alcohol/ethyl alcohol to remove the lesions, which is diagnostic and therapeutic too. This procedure possibly determines protein denaturation, interfering in the cell metabolism and diluting lipoprotein membranes,

 thus avoiding blood tests and skin biopsies. Mild inflammation, erythema, stinging-like sensation are reported after vigorous rubbing of repeated applications. The skin should be washed with soap and water after using alcohol, considering the possible symptoms of intoxication as drowsiness, lethargy, respiratory depression, mucosal irritation and others, especially in children. Other keratolytic agents like salicylic acid 5%/20% in alcohol base, 30% Urea, 12% Ammonium lactate showed similar efficacy after application for 2 weeks on the affected area, without any irritation/erythema.

 Moisturizing the affected skin is important to prevent xerosis after regular treatments with isopropyl alcohol. Recurrence is unusual after proper treatment. Rare recurrences can also be treated weekly with alcohol wipes or topical keratolytic agents.

At follow-up 3 months later there were no signs of recurrent pigmentation, and he did not require any cleaning with alcohol wipes.

1. Aslan NC, Guler S, Demirci K, Isiyel E. Features of terra firma- forme dermatosis. Ann Fam Med., 2018 Jan; 16(1):524

2. Guarneri C, Guarneri F, Cannova SP. Terra firma – forme dermatosis. Int J. Dermatol., 2008; 47:482-484

3. Panchal K, Bhalla N, Salunke P,Jerajani H. Extensive terra firma forme dermatosis (TFFD): A rare presentation. Indian Dermatol Online J. 2015 Nov- Dec;6(6):458–459

4. Abdel-Razak MM, Fathy H. Terra firma – forme dermatosis: case series and dermascopic features. Dermatol Online J. 2015;21(10)

5. Greywal T, Cohen PR. Noninvasive methods to establish the diagnosis of terra firma – forme dermatosis: the SMART (skin modified by alcohol rubbing test) evaluation and dermoscopy. Dermatol Online J. 2016;22(6):19

6. Sasaya EMK, Ghislandi C, Trevisan F, et al. Dermatosis neglecta. An Bras Dermatol. 2015; 90(3 suppl 1):59 – 61

7. Leung AKC, Lam JM. Erythema dyschromicum perstans in an 8 year – old Indian child. Case Rep Dermatol Med. 2018. doi:10.1155/2018/2143089

8. Leung, AKC, Barankin B, Leung, AAM, Lam JM. A hyperpigmented patch that remains despite washing with soap and water. Consultant 360. 2018;5 (11):309 – 312, 315

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10. Chun SW, Lee SY, Kim JB, Choi HM, Ro BI, Cho HK. A case of Terra firma – forme dermatosis treated with salicylic acid alcohol peeling. Ann Dermatol. 2017;29(1):83- 85

Articles

During the evaluation of a healthy 7-year-old boy, an asymptomatic brown “dirt-like” rash is found on the anterior neck. It has progressed over the last 2 months and does not clear with routine bathing and aggressive scrubbing by his mother with soap and water. 

A case of terra firma—forme dermatosis

A 17-month-old African American girl, with no significant past medical history, is brought to the Pediatric Emergency Department (PED) with acute onset of swelling in the floor of her mouth. The mother is a reliable historian who denies any recent illness, fever, travel, change in food/new food intake, drugs, or significant injury/trauma.

The girl was in her usual state of health when the mother dropped her at the daycare in the morning. However, after returning home from daycare in the evening, the mother noticed her daughter to be more irritable, drooling, and refusing to eat. The mother also noted a bright red-colored swelling underneath the child’s tongue (Figure 1), which prompted this PED visit. The mother denies any visible rashes, new skin lesions, bleeding, bloody stools, diarrhea, or dark-colored urine.

On oral exam, bright erythematous, nontender, firm masses were seen underneath the patient’s tongue on both sides of the frenulum area (Figure 1). Mild bleeding was noted from the swelling. Each swelling was 2- to 3-cm in size and located underneath the tongue, appearing firm, noncompressible, and symmetrical in size on either side of the frenulum. Some blood-stained saliva was noted over the swelling. Elevation and bright red-colored swelling under the oral mucosa were consistent with the hematoma noted.

Sublingual caruncles looked swollen and elevated. There was no evidence of any wheezing, stridor, or difficulty with respiration. On examination of the neck, clinicians noted a small swelling in the midline that was soft, fluctuant, and tender to touch, located just behind the chin (Figure 2).

Blood workup showed: white blood cell (WBC) count, 12.5; hemoglobin, 12.4 g; aspartate transaminase (AST)/alanine transaminase (ALT), 33/15; erythrocyte sedimentation rate (ESR), 19 mm/h; uric acid, 1.5 mg/dL; lactate dehydrogenase (LDH), 304 IU/L; C-reactive protein (CRP), 1.04 mg/dL; prothrombin time (PT), 12.8 sec; activated partial thromboplastin (aPTT), 34 sec; international normalized ratio (INR), 1; fibrinogen, 407 mg/dL.

Based on the above results, the possible differential diagnoses of oral cavity swellings in the floor of the mouth are numerous (Table):

Ludwig angina is a bilateral infection of the submandibular space including sublingual and submylohyoid (submaxillary) space. It is characterized by brawny induration of the floor of the mouth with an elevation of the tongue, potentially obstructing the airway. Ludwig angina is up to 90% odontogenic in origin. Other causes are peritonsillar/parapharyngeal abscess, oral lacerations, and mandibular fractures. Fever, neck swelling, bilateral submandibular swelling, and elevation of the tongue are the common mode of presentation. The most common etiologies are aerobes, anaerobes, alpha-hemolytic streptococci, 

. High-dose intravenous (IV) penicillin, clindamycin, and metronidazole are the best agents for infection. Complications are sepsis, pneumonia, asphyxia, and respiratory obstruction.

Ranula is a mucous retention pseudocyst in the floor of the mouth. It can originate from trauma or obstruction of the sublingual salivary glands, less commonly submandibular/minor salivary glands may be involved. Oral ranulas originate superior to mylohyoid muscle. The intraoral component usually has a bluish color and is typically unilateral, although bilateral swelling can occur. Complete excision of the pseudocyst with the affected salivary gland is associated with the least likelihood of recurrence.

 Cervical/plunging ranulas that penetrate through the belly of the mylohyoid muscle often produce an externally visible neck mass. Posttraumatic ranula will contain mucin in the aspirate. Other modalities of treatment are marsupialization, cryosurgery, and carbon dioxide laser excision.

The oral cavity/neck is a potential target for many injuries. Penetrating injuries secondary to metal bodies/dentures/foreign bodies/metal hooks can be a serious cause of lingual/sublingual hematoma. Few case reports of coat hanger-,

 fishhook-induced oral trauma leading to soft tissue swellings are reported. Infected sublingual hematoma secondary to tongue-tie surgery is reported in the literature as sublingual swelling.

Approximately 50% to 60% of patients with severe hemophilia (factor level <1% of the factor activity) usually present after oral trauma. It is secondary to dental surgeries, penetrating trauma, and lobsters. Food-induced oral injury can lead to acute bleeds and cause rapid worsening of sore throat, breathing difficulties, and stridor. Prompt treatment with factor 8 concentrates repeated for every 12 hours and close monitoring of vitals and respiratory status are strongly warranted. Early diagnosis and prompt treatment with factor-8 concentrates can protect airways and prevent intubations/surgical management.

Sublingual hematoma is a rare complication of excessive anticoagulation. It can mimic Ludwig angina, often called pseudo-Ludwig phenomenon. Acute onset of sore throat, swelling in the floor of the mouth, drooling, stridor at rest, and difficulty with speaking in a patient on oral warfarin strongly suggests sublingual/submandibular hematoma. Lack of signs of cellulitis and normal inflammatory markers with elevated PT/INR are suggestive of a warfarin-induced hematoma. Sublingual hematomas typically develop quickly and extend posteriorly to involve the supraglottic larynx. Coagulation abnormality can be effectively corrected with fresh frozen plasma, IV vitamin K, and prothrombin complex concentrates.

 Acute lingual/sublingual hematoma secondary to antipsychotic haloperidol drug use has been reported. Acute dystonic reaction involving the head and neck can lead to oral trauma and acute sublingual hematoma.

Dermoid cysts of the floor of the mouth are rare lesions caused by entrapment of germinal epithelium during the closure of the mandibular and hyoid branchial arches. The histological varieties are: epidermoid, if the epidermis is lining the cyst; dermoid, if skin annexes exist; and teratoid, derivatives of 3 germinal layers. A dermoid cyst usually presents as a smooth, doughy, lobular, compressible cystic mass on the floor of the mouth. The cyst lumen is filled with a mixture of desquamated keratin and sebum.

 Complete surgical excision of the lesion using either intraoral or extraoral approach is the best treatment of choice. Magnetic resonance imaging (MRI) of the neck in this patient shows a high-intensity mass on the T2-weighted images of dermoid cyst.

Teratomas are exceptionally rare tumors in the head and neck region. Neonatal teratomas occur in about 1:20,000 to 1:40,000 live births. Most congenital teratomas are mature or immature ones but they can recur as malignant teratoma after initial surgery.

 Teratomas are tumors containing components of all 3 embryologic germ layers originating from multipotent germ cells. The incidence of malignancy of sacrococcygeal teratoma in neonates is approximately 10% and reaches 100% by age 3 years. Malignant transformation of a teratoma is documented by rapid growth after excision with markedly elevated alpha-fetoprotein (AFP) levels. It can be curable with multi-agent chemotherapy along with complete resection. The residual malignant component can be monitored by serial AFP measurements and complete resection is assured by the decline of AFP to normal after cessation of the chemotherapy.

Foreign bodies are an unusual cause of a slow-growing mass at the floor of the mouth. Fishbones, metal hooks, broken teeth fragments, parts of dentures, pen caps,

 and air rifle pellets are rarely diagnosed and can present with a slow-growing mass in the oral cavity. After the initial injury, the open wound of the floor of the mouth can act as a check valve mechanism that leads to pneumatocele leading to slow growth.

Physical abuse in infants and children is a rare cause of sublingual hematoma. Most common injuries are tears of the lingual frenulum and open wounds of the floor of the mouth, gingiva, and palate.

 Metaphyseal bone fractures, rib fractures, various skin bruises, and retinal hemorrhages are other clinical findings of child abuse. Bleeding diathesis, osteogenesis imperfect, cutis laxa, and Ehlers-Danlos syndrome should be ruled out before the diagnosis of child abuse.

Based on the patient’s clinical history, physical examination, and the acuity of presentation, the clinicians suspected traumatic swelling on the floor of the mouth, probably an acute swelling of sublingual/submandibular salivary glands, as the most common possible etiology. The girl was admitted on the Pediatric floor for close follow-up of the swelling, work of breathing, and oral intake, and close monitoring for any acute worsening of stridor and desaturations. Oral Surgery and Otolaryngology were consulted for further recommendations and management.

On repeat exam after a few hours, clinicians noticed that the color of the swelling changed to dark purplish color (Figure 3). Based on the acuity of presentation, color change of the hematoma, mild bleeding from the swelling, and midline acute tender fluctuant swelling, they strongly suspected oral trauma in this case. As the mother denied any significant trauma, they communicated with the daycare center staff for further clarification and inquired about any possible oral trauma/fall/significant injury during the child’s daycare stay. The staff confirmed that the girl had fallen with a toy (Figure 4) in her mouth/neck while she was running in the daycare.

The Oral Surgery team confirmed final diagnosis as bilateral sublingual hematoma secondary to oral trauma. Oral cavity swellings usually represent engorged sublingual salivary glands with blood/hematoma.

The patient was closely monitored for any worsening of swelling size and development of stridor and drooling. Care continued with warm compressions and a soft, liquid diet as tolerated. She remained stable at room air with stable vitals.

The patient was discharged in stable condition with instructions for warm compressions and close follow-up instructions with Oral Surgery on an outpatient basis. She was seen after 1 week and her oral swellings were completely resolved without any residual complications or adverse events reported.

1. Srirompolong S, Art-Smart T. Ludwig’s angina: a clinical review. 

2. Zhao YF, Jia J, Jia Y: Complications associated with surgical management of ranulas. 

3. Cheng J, Kleinberger A, Dunham B, Woo P. Do not hang your coat here. 

4. Kausar H, Gilani JM, Khan OA. No more Doritos and lobster tails: a case report of life-threatening sublingual hematoma. 

5. Spindler T, Mc Goldrick N, McMahon J, Campbell Tait R. Spontaneous sublingual haematoma in acquired haemophilia: case report. 

6. Lim M, Chaudhari M, Devesa PM, Waddell A, Gupta D. Management of upper airway obstruction secondary to warfarin therapy: the conservative approach. 

7. Lima SM Jr, Chrcanovic BR, de Paula AM, Freire-Maia B, Souza LN. Dermoid cyst of the floor of the mouth. 

8. Ueno S, Hirakawa H, Matsuda H, et al. A case of neonatal mature teratoma transformed to malignancy in the neck extending to the mouth floor. 

9. Uguz MZ, Kazikdas KC, Erdogan N, Aydogdu V. An unusual foreign body in the floor of the mouth presenting as a gradually growing mass. 

10. Kudek MR, Knox BL. Sublingual hematoma: when to suspect child abuse. 

A 17-month-old African American girl, with no significant past medical history, is brought to the Pediatric Emergency Department with acute onset of swelling in the floor of her mouth. What's the diagnosis?

Swayam P Nirujogi, MD, MBA

Articles

A case of terra firma—forme dermatosis

Toddler’s unusual oral trauma is troubling