A 15-year-old boy with a bilateral plantar rash and an inability to bear weight 1 day after engaging in strenuous physical activity is admitted to the hospital pediatrics service. The rash consists of erythematous, blanching macules and patches with distribution along the weight-bearing aspects of the toes, soles, heels, and lateral aspects of the feet (Fig 1). It is painful and radiates along the posterior legs toward the knees. He has had 2 days of intermittent fevers (100.6°F–101.8°F). During febrile episodes, the rash becomes more erythematous and painful. Activities performed 2 days prior to admission include 6 hours of football practice, weight-lifting and cardiovascular exercise, which is more intensive than his usual routine. The patient reports a history of hyperhidrosis but denies any history of other dermatoses or vasculitis. He denies any recent travel or engagement in sexual activity.On physical examination, the bilateral plantar aspects of the feet are warm and exquisitely tender to palpation. There is normal range of motion of the ankle and toes. Sensation to light touch is intact. Dorsalis pedis and posterior tibial pulses are 2+. Capillary refill is less than 2 seconds. Laboratory evaluation shows a white blood cell count of 17 K/μL (reference range: 4.5–13.5 K/μL) and a C-reactive protein level of 6.1 mg/dL (reference range: <1.2 mg/dL). Infectious evaluation, including influenza A/B antigens, Epstein-Barr virus IgM, group A streptococcus polymerase chain reaction, respiratory viral panel, human immunodeficiency virus antibodies, rapid plasma reagin, chlamydia and gonorrhea polymerase chain reaction, urinalysis, and blood cultures are unremarkable. Immunologic evaluation, including antineutrophil cytoplasmic antibody, myeloperoxidase, and proteinase 3 antibodies, rheumatoid factor, C3, and C4 levels are similarly unremarkable. Radiographs of the bilateral ankles and feet and magnetic resonance imaging of the right foot are normal. The dermatology service is consulted, and a punch biopsy is taken from a lesion on the right foot.Dermatopathological analysis of the biopsy (Fig 2) shows a dermal infiltrate composed of neutrophils and lymphocytes in the superficial and deep dermis surrounding the eccrine glands and extending into the perivascular space and subcutaneous fat without metaplasia. Focal abscesses were noted in the deep dermis. Physical examination and pathology are consistent with palmoplantar eccrine hidradenitis.Palmoplantar eccrine hidradenitis was initially described in the literature by Stahr et al. (1) Patients experienced tender, erythematous nodular and papular lesions on their soles. Laboratory evaluation was noncontributory, and constitutional symptoms were generally absent. Given the histologic similarity to neutrophilic eccrine hidradenitis, a condition affecting mainly chemotherapy patients and characterized by neutrophilic infiltration of the eccrine coil with necrosis and possible squamous metaplasia, Stahr et al (1) named this condition idiopathic plantar hidradenitis. Rabinowitz et al (2) subsequently identified 2 patients with similar painful, nodular lesions also affecting the palms. Due to the tendency of the lesions to recur, the condition was renamed as recurrent palmoplantar hidradenitis. (2)Palmoplantar hidradenitis characteristically presents as multiple, tender, erythematous nodules and occasional pustular formations on the palms or soles causing intense pain that interferes with weight-bearing activities. Fever (1)(3)(4)(5)(6) and elevated inflammatory markers (2)(5) are reported in some cases. Inciting events include mechanical trauma, (7)(8)(9) prolonged water exposure, (2)(6)(10) and a history of hyperhidrosis. (3)(11) Histopathology shows an intense neutrophilic infiltrate affecting the eccrine apparatus, most notably the coils, located in the dermal layer with possible abscess formation (1)(2)(4) and extension into the perivascular space (1)(2)(5)(11) or subcutaneous fat layer. (1)(2)(5) It is hypothesized that the impact of mechanical trauma in combination with the obstruction that results from excess sweat production (3) causes rupture of the eccrine glands. Subsequent activation of the signaling cascade and cytokine release leads to neutrophilic attraction to the site of rupture, (12) resulting in the histopathologic features described.The differential diagnosis includes several other etiologies. Erythema nodosum presents as painful nodules affecting the anterior shins. Histology reveals panniculitis. Acute febrile neutrophilic dermatosis, also known as Sweet syndrome, presents with diffusely distributed painful pustules, papules, and nodules, which show dense neutrophilic infiltrate without vasculitis on histology. Neutrophilic eccrine hidradenitis, as described above, shares similar histologic findings with palmoplantar eccrine hidradenitis; however, sweat glands show evidence of syringosquamous metaplasia. Traumatic pressure urticaria results from mechanical stress but shows no evidence of eccrine neutrophilic infiltration. Chilblains commonly occur after exposure to cold, damp environments, but these lesions typically appear as plaques and patches with microscopic evidence of perivascular lymphocytic infiltrate. “COVID toes” refers to chilblainslike, erythematous or purple, purpuric lesions affecting the distal extremities with macular, plaquelike, nodular, or vesicular eruptions associated with severe acute respiratory syndrome coronavirus 2. (13)Our patient demonstrated several risk factors for palmoplantar hidradenitis given his recent history of strenuous physical activity and chronic hyperhidrosis. The distribution of his rash was consistent. Still, several unique features delayed his diagnosis. The apparent systemic involvement of his rash evidenced by leukocytosis and elevated inflammatory markers, as well as febrile episodes accompanied by severe diaphoresis and associated exacerbation of symptoms, was misleading. Other cases reporting on a fever described it as mild (1)(4) or low-grade. (5) Due to these systemic signs and symptoms and the rarity of this condition among pediatric patients, a large infectious and immunologic evaluation was performed. Systemic diseases that involve a palmoplantar eruption include Rickettsial diseases, such as Rocky Mountain Spotted Fever, atypical hand foot and mouth disease caused by coxsackievirus, disseminated intravascular coagulation, meningococcal meningitis, secondary syphilis, parvovirus B19 rarely presenting with a pruritic, plantar eruption, and infective endocarditis with embolic phenomena.The clinical course of palmoplantar eccrine hidradenitis is typically benign with resolution in days to weeks. Recurrence is seen in up to 50% of cases (12) with 1 case reporting yearly relapses. (11) Management is supportive and consists of analgesics and bed rest. The use of oral or topical corticosteroids has been reported, (1)(3)(4)(6) but not formally studied.Palmoplantar eccrine hidradenitis is mainly described in the dermatologic literature; however, these patients typically first present to primary care clinics, urgent cares, and emergency departments. Given recent reports suggesting that this form of hidradenitis may be more common than previously thought, (3) there is a need to further the knowledge of clinicians involved in the initial evaluation to prevent extensive laboratory testing and imaging, specialty consultation, and unnecessary or lengthy hospitalization.On admission, our patient was treated with acetaminophen and ibuprofen for relief of pain and fever control. Following biopsy acquisition, he was started on 0.05% topical clobetasol. The following morning, he reported improvement in pain and was able to bear weight. He was discharged on hospital day 5 after remaining afebrile over a 24-hour period. He was seen in the dermatology clinic 2 weeks after discharge and had complete resolution of his symptoms.
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Jan 5, 2024
Alessandra Iurlo + 5