Scarring Alopecia of Scalp

It is Not Always About the Hair!

Dear Editor,

Sarcoidosis is an idiopathic granulomatous disorder with cutaneous and extracutaneous manifestations. Cutaneous manifestations in sarcoidosis can be specific or non specific to the disorder and are polymorphic in nature. Scalp sarcoidosis is one the rare cutaneous presentations of this disorder and mimics common causes of scarring alopecia of scalp. We present a rare of scalp sarcoidosis manifesting as scarring alopecia.

Our patient, 63 years old female presented with complaints of localized patch of hair loss with redness and flaking over scalp of one year duration. The complaint was gradually progressive with increase in the size of alopecic patch along with appearance of new patches. She also had associated mild pain and occasional pruritus over the site of hair loss. Patient was a known case of type 2 diabetes mellitus, dyslipidemia, hypertension and hypothyroidism on medication. Examination of the scalp revealed a linear well- defined plaque with scarring alopecia with areas of erythema, crusting and erosion over the parietal scalp of size 5 cm × 2cm. [Figure 1 and Figure 2] Systemic examination revealed no abnormalities in any other organ system. A provisional diagnosis of cicatricial alopecia was made and a punch biopsy was done to differentiate between the various clinical differentials in the form of discoid lupus erythematosus, pemphigus foliaceous, Bowen’s disease and linear morphea.

Figure 1: Linear well defined plaque with scarring alopecia with areas of erythema, crusting and erosion over the parietal scalp of size 5x2cm

Figure 2: Crusted plaque over the alopecic scalp

Dermoscopy of the scalp lesion revealed scarring alopecia with thick dilated tortuous vessels. Honeycomb pattern of pigmentation with occasional thick black crusts entangling the hair root (star burst pattern) were seen. No follicular plugging was present [Figure 3].

Figure 3: Dermoscopy of the scalp lesion revealed scarring alopecia with thick dilated tortuous vessels. Honeycomb pattern of pigmentation with occasional thick black crusts entangling the hair root (star burst pattern) were seen

Histopathological examination of the punch biopsy from the lesions showed numerous discrete non caseating epitheloid cell granulomas with numerous interspersed langhan’s and foreign body type of giant cells with lymphocytes. Occasional intracytoplasmic star shaped eosinophilic inclusions, morphologically consistent with asteroid bodies were seen. No follicular structures were evident. [Figures 456] On the basis of these histological findings, a provisional diagnosis of sarcoidosis of scalp was made.

Figure 4: Histopathological examination showed numerous discrete non caseating epitheloid cell granulomas with numerous interspersed langhan’s and foreign body type of giant cells with lymphocytes

Figure 5: Histopathology seen at a higher power, collection of epithelial cells can be seen with sparse lymphocytes(H&E;100X)

Figure 6: Eosinophilic asteroid bodies can be seen on high power.(H&E;100X)

ACE and serum calcium levels were in normal range, 36.39 U/l (12–68 U/l) and 9.23 mg/dl (8.6 -10.2 mg/dl). Haemoglobin was 10.7 mg/dl, which showed dimorphic anaemia on peripheral blood smear. No abnormalities were found on the chest roentgenogram.

Patient was diagnosed as a case of scalp sarcoidosis based on clinical, dermoscopic and histopathological findings. She was started on oral steroids along with hydroxychloroquine with good response to therapy.[Figure 7]

Figure 7: Post treatment response picture showing improvement in the alopecia and crusting

Sarcoidosis is a multi-system idiopathic disorder with cutaneous sarcoidosis occurring in almost 25% cases of systemic sarcoidosis. This clinical condition morphologically exhibits varied presentation, thus imitating many unrelated dermatologic conditions. Hence, it has been described as a great mimicker in dermatology.[¹] Scalp sarcoidosis is a rare clinical entity causing cicatricial alopecia, most commonly encountered in African-American women. The disease shows a strong gender bias with female preponderance.[²] Our case also followed this trend. Diagnosis is made primarily by histopathology along with clinical features.[³] Sarcoidal alopecia has classic non caseating sarcoidal granulomas in dermis, also called naked granulomas because of a sparse lymphocytic infiltrate surrounding the granuloma.[⁴] A number of giant cells of Langhan’s and foreign body type can also be seen. The dermis can show marked fibrosis with destruction of follicular structures. Occasional intracytoplasmic star shaped eosinophilic inclusions, morphologically consistent with asteroid bodies can be seen.[⁵] Our case demonstrated all the typical histopathological changes of sarcoidosis.

Early diagnosis of sarcoidosis is important as it involves the risk of multisystemic involvement. Approximately 30% of patients with cutaneous sarcoidosis will develop systemic manifestations within months to years of diagnosis.[³]

Therapy of scalp sarcoidosis with alopecia has been quite unsatisfactory. Various reports of therapy of scalp sarcoidosis with corticosteroids (topical and intralesional and systemic), antimalarials, methotrexate and infliximab have shown poor to moderate response.[²] Our case showed good response to treatment with oral and topical corticosteroids along with hydroxychloroquine with cessation of disease progression.

Not much is known about the overall prognosis of scalp sarcoidosis primarily because of the small number of reported cases. It might be possible that many cases of cicatricial alopecia due to sarcoidosis are often misdiagnosed and left untreated in the absence of proper histopathological evaluation.

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