Martin A. Coles, MD

For 2 days, an 11-month-old girl had a progressively worsening rash and subjective fever (

). The rash began on the legs as bumps, which later became large violaceous lesions (

) and spread to the face, arms, and trunk. During the 12 hours before presentation, erythematous swelling of the periorbital area and ears developed. The upper eyelid swelling was the mother's greatest concern because it limited the baby's ability to open her eyes. The infant had had no difficulty in breathing and no emesis, diarrhea, or changes in urinary output. She had been eating less but drinking well.

The patient was alert and crying but consolable. Vital signs were normal. Oxygen saturation was 100% on room air. Weight and height were appropriate for age. Pulmonary, cardiovascular, GI, and neurological findings were normal. Multiple palpable, purpuric lesions were noted on the face, ears, trunk, and upper and lower extremities. The facial lesions were not warm, tender, or blanching. There was significant edema of the upper eyelids, pinnae, and ear lobes with dark ecchymosis. Intraoral findings were normal.

Laboratory results, including erythrocyte sedimentation rate, C-reactive protein level, coagulation factors, chemistry panel with liver enzyme levels, urinalysis, complete blood cell count, and platelet count, were all normal. Stool was guaiacnegative. Results of a rapid streptococcal screen were positive.

The infant was given 1 dose of intravenous methylprednisolone for suspected Henoch-Schnlein purpura (HSP) and 1 dose of oral diphenhydramine hydrochloride because of the possibility of abdominal or joint pain, which may have been present but which the infant was unable to communicate. Mild improvement of the eyelid swelling was noted the next day (

Acute hemorrhagic edema of infancy (AHEI), also known as Finkelstein or Seidlmayer disease, was diagnosed. Although some experts feel that this condition is a variant of HSP, others believe that it is a separate disease. If AHEI is a variant of HSP, one would expect that it would be similarly mediated by immune complexes. However, IgA deposition on biopsies of skin lesions is less common in AHEI than in HSP. Also, unlike HSP, AHEI typically occurs in children aged 4 to 24 months. As in HSP, presentation is often dramatic-as was the case in this infant. Often, patients have a recent history of an upper respiratory tract infection or ear infection.

AHEI is a cutaneous vasculitis that usually presents with palpable purpura or petechiae primarily on the face, ears, and extremities, similar to HSP. The first lesions appear as small macules or papules, which the family often confuses with bug bites. Over the next few days, the lesions enlarge. Edema of the face and lower and upper extremities may also develop. The arthritis/arthralgia, nephritis, abdominal pain, and GI bleeding that accompany HSP are rare in AHEI. Although routinely benign, AHEI needs to be distinguished from more serious conditions, such as meningococcemia, Rocky Mountain spotted fever, erythema multiforme, child abuse, Kawasaki disease, and septicemia.

AHEI usually resolves spontaneously within 1 to 3 weeks. The most worrisome complication is necrosis of the structures of the outer ear, especially the ear lobe, which occasionally develops in the areas of severe swelling and purpura. Treatment is symptomatic. The use of corticosteroids is controversial and not universally recommended. Although it rarely happens, the disease can recur.

This little girl presented with a classic case of AHEI. She appeared nontoxic, despite the impressive swelling of her face and ears with the purpuric rash. She recovered quickly with almost complete resolution of the rash about 4 days after presentation (

Â Can B, Kavala M, TÃ¼rkoglu Z, Zemheri E. Acute hemorrhagic edema of infancy: a case report. Turk J Pediatr. 2006;48:266-268.

Â da Silva Manzoni AP, Viecili JB, de Andrade CB, et al. Acute hemorrhagic edema of infancy: a case report. Int J Dermatol. 2004;43:48-51.

Javidi Z, Maleki M, Mashayekhi V, et al. Acute hemorrhage edema of infancy. Arch Iran Med. 2008;11:103-106.

Shetty AK, Desselle BC, Ey JL, et al. Infantile Henoch-SchÃ¶nlein purpura. Arch Fam Med. 2000;9:553-556.

Articles

For 2 days, an 11-month-old girl had a progressively worsening rash and subjective fever (A). The rash began on the legs as bumps, which later became large violaceous lesions (B) and spread to the face, arms, and trunk.

Acute Hemorrhagic Edema of Infancy in an 11-Month-Old Girl

A 10-year-old boy had a 3-day history of an itchy eye. His mother thought he had somehow cut his upper left eyelid.

The boy had no history of recent trauma or discharge from the affected eye. He denied eye pain, swelling, and changes in vision. He was afebrile and otherwise healthy. Previous medical history was noncontributory.

The patient's sclerae were clear; his pupils were equal, round, and reactive to light. Extraocular movements were intact. A flattened, glistening, pink, polypoid mass on a thin stalk protruded from the inner aspect of the lateral upper left eyelid.

Martin Coles, MD, of Pediatric Emergency Medicine Associates, Atlanta, suspected pyogenic granuloma. A pediatric ophthalmologist who reviewed the case agreed with the presumptive diagnosis. The child was referred to the ophthalmologist for excision of the lesion. However, he subsequently moved to Mexico and in a follow-up phone conversation, the mother stated that the lesion had resolved on its own after a few weeks. The clinical presentation of the lesion and its spontaneous regression without intervention strongly suggest pyogenic granuloma.

The term "pyogenic granuloma" is a misnomer: this lesion is neither infectious nor histologically granulomatous. Its cause is unknown. Pyogenic granulomas usually appear in late childhood. The majority of these benign masses appear on the head, neck, and extremities; lesions on the conjunctiva are unusual, however. The differential diagnosis of such lesions includes squamous papilloma, conjunctival lymphoma, and ocular lymphangiectasis. Pathologic examination is necessary for definitive diagnosis.

Pyogenic granulomas grow rapidly and regress spontaneously. Removal is indicated for cosmesis or when the mass causes discomfort or is prone to bleeding. Simple excision at the base of the lesion is the treatment of choice. Pathologic examination of the specimen after removal is recommended because these lesions can mimic malignant tumors, such as squamous cell carcinomas. The lesions may recur after removal.

Conjunctival "Pyogenic Granuloma"

14-Year-old boy who presents to emergency department with nonpainful swelling under his chin that began about a week earlier. Swelling has been gradually growing; patient now has some difficulty in flexing his neck.

Patient appears otherwise healthy and has no significant medical history. No recent fever or trauma. Vital signs, including blood pressure, normal.

Large, soft, right-sided mass, palpable from the tip of chin down to the level of the cricoid cartilage. Not warm, red, or tender to palpation. No movement of the mass with swallowing; it does not cross the midline. Patient is not drooling and has no respiratory difficulty. Posterior oropharynx,soft palate, and dentition all normal. Patient can open his mouth without difficulty.

Examination of the underside of the tongue reveals a blue cystic mass on the floor of the mouth. This right-sided mass appears to be fluid-filled: no pus, blood, or debris apparent.

 are pseudocysts that develop when mucus leaks into surrounding tissues. They arise from major salivary glands; mucoceles arise from minor salivary glands. Both mucoceles and ranulas may occur secondary to trauma or to blocked salivary excretion.

There are 2 types of ranulas: oral and plunging.

Usually painless, and frequently asymptomatic, 

can sometimes cause problems with oral function--including speech, swallowing, and occasionally, respiration. An oral ranula appears as a blue-hued translucent swelling on the floor of the mouth that resembles the belly of a frog.This rare fluid-filled mass is compressible and painless.

 Usually seen coincidentally with an oral ranula (as with this patient), a plunging ranula can arise without the blue translucent swelling at the floor of the mouth.

Needle aspiration may be therapeutic; however, swelling generally recurs. Other methods of treatment include marsupialization as well as excision of the ranula and the offending sublingual gland.

 In one study, the recurrence rates with these methods were 66.67%, 57.69%, and 1.2%, respectively.

This patient initially underwent marsupialization. The lesion recurred about 4 months later. Ultimately, the ranula was excised and the involved sublingual gland was obliterated. The patient is currently doing well.


1.Langlois NE, Kolhe P. Plunging ranula: a case report and a literature review. 

 1992;23:1306-1308.
2.Morita Y, Sato K, Kawana M, et al. Treatment of ranula--excision of the sublingual gland versus marsupialization. 

 2003;30:311-314.
3.Zhao YF, Jia Y, Chen XM, Zhang WF. Clinical review of 580 ranulas. 

Oral Surg Oral Med Oral Pathol Oral Radiol Endod.

Patel NJ, Sciubba J. Oral lesions in young children. 

Author | Martin A. Coles, MD

Articles

Acute Hemorrhagic Edema of Infancy in an 11-Month-Old Girl

Conjunctival "Pyogenic Granuloma"

Ranulas in a 14-Year-Old Boy