SKINDIA QUIZ 1: Erythematous, firm, papular eruptions involving the face, neck and chest.

A 24-year-old man presented to the outpatient department with multiple erythematous, firm, papular eruptions, involving the face, neck and chest, of nearly 2 weeks duration. The lesions erupted in crops and were largely asymptomatic. The eruptions healed with scarring. A skin biopsy was done from the neck.

ANSWER

Diagnosis

FIGURE (Facial Idiopathic Granulomas with Regressive Evolution), previously termed lupus miliaris disseminatus facei/ acne agminata

DISCUSSION

FIGURE or acne agminata is a disorder characterized by the eruption of multiple, monomorphic, symmetrical, reddish brown papules of the chin, forehead, cheeks and eyelids. The lesions tend to erupt in crops [Figures 1 and 2]. It is common in adolescents and young adults of either gender. The lesions may cluster around the mouth and the eyelids and hence the term “agminata” seems appropriate. Paradoxically, the lesions may be widely disseminated around the face and the term “disseminatus” may seem more applicable. [1] Extrafacial lesions have been reported but are rare. The disorder is self-limiting and resolves completely within a few months to 2 years. In some cases, there is scarring.

Figure 1

Crops of multiple, erythematous, monomorphic, firm, papular eruptions

Figure 2

A closer view demonstrates the lesional morphology. The lesion is firm in consistency

The etipathogenesis of this distinct entity is controversial. A tubercular etiology was initially proposed based on the classical histopathology of a granulomatous reaction with a central caseation necrosis. The disease was hence classifed as a distinct presentation of tuberculosis. The name lupus miliaris disseminates facei originated from this contention of it being tuberculous in etiology.

It was then perceived to be a self-limiting variant of granulomatous rosacea. However, there are arguments proposed against this line of thought too.

FIGURE is a recently proposed acronym for “Facial Idiopathic Granulomas with Regressive Evolution” and this term excludes the possibility that this is either of tubercular origin or is a variant of acne. [2]

This condition is often self-limiting and unresponsive to treatment. A skin biopsy was done from the neck for the 24-year-old man who presented with the symptoms of FIGURE. The photomicrographs are shown [Figures 3 and 4].

Figure 3

Histopathology showing an unremarkable epidermis and a dermal infiltrate with a granulomatous response (H&E, ×10)

A higher power shows the granulomatous response better (H&E, ×40)

Drugs tried in its treatment include Isotretinoin, [1] Dapsone, [3] low dose prednisolone [4] and Clofazamine. [5]